PXD010028

PXD010028 is an original dataset announced via ProteomeXchange.

Title	Effects of 4-phenylbutyrate on human keratinocytes
Description	Keratins 5 and 14 are critical for cytoskeletal integrity, as shown by missense mutations in these genes, which cause the severe skin fragility disorder epidermolysis bullosa simplex (EBS). The complexity of the pathomechanisms in EBS is not fully understood and no effective management exists. In addition to fragility, EBS keratinocytes are characterized by aggregates of misfolded keratin. Here, we tested the chemical chaperone 4-phenylbutyrate (4-PBA) as a putative novel therapy, using keratinocytes from patients with severe generalized EBS due to distinct KRT5 and KRT14 mutations.
HostingRepository	PRIDE
AnnounceDate	2019-12-03
AnnouncementXML	Submission_2019-12-03_02:45:21.xml
DigitalObjectIdentifier
ReviewLevel	Peer-reviewed dataset
DatasetOrigin	Original dataset
RepositorySupport	Unsupported dataset by repository
PrimarySubmitter	Joern Dengjel
SpeciesList	scientific name: Homo sapiens (Human); NCBI TaxID: 9606;
ModificationList	No PTMs are included in the dataset
Instrument	LTQ Orbitrap

Revision	Datetime	Status	ChangeLog Entry
0	2018-06-05 00:34:15	ID requested
⏵ 1	2019-12-03 02:45:58	announced

Sp, ö, rrer M, Prochnicki A, T, ö, lle RC, Nystr, ö, m A, Esser PR, Homberg M, Athanasiou I, Zingkou E, Schilling A, Gerum R, Thievessen I, Winter L, Bruckner-Tuderman L, Fabry B, Magin TM, Dengjel J, Schr, ö, der R, Kiritsi D, Treatment of keratinocytes with 4-phenylbutyrate in epidermolysis bullosa: Lessons for therapies in keratin disorders. EBioMedicine, 44():502-515(2019) [pubmed]

curator keyword: Biomedical

submitter keyword: human, skin, epidermolysis bullosa, keratinocytes, LC-MS/MS

Joern Dengjel
contact affiliation	Department of Biology, University of Fribourg Chemin du Musée 10, 1700 Fribourg, Switzerland T +41 26 300 8631 F +41 26 300 9741
contact email	joern.dengjel@unifr.ch
lab head
Joern Dengjel
contact affiliation	University of Fribourg
contact email	joern.dengjel@medizin.uni-freiburg.de
dataset submitter

Dataset FTP location NOTE: Most web browsers have now discontinued native support for FTP access within the browser window. But you can usually install another FTP app (we recommend FileZilla) and configure your browser to launch the external application when you click on this FTP link. Or otherwise, launch an app that supports FTP (like FileZilla) and use this address: ftp://ftp.pride.ebi.ac.uk/pride/data/archive/2019/12/PXD010028

PRIDE project URI

• PRIDE
  1. PXD010028
     1. Label: PRIDE project
     2. Name: Effects of 4-phenylbutyrate on human keratinocytes